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Background: Cutaneous nodular amyloidosis is rare, and association with Sjögren's syndrome has been reported. We discuss the possible link between these two diseases based on a case we saw., Patients and Methods: A 78-year-old woman with Sjögren's syndrome ongoing for 17 years presented for evaluation of a waxy infiltrated lesion on her left leg that had appeared 6 months earlier. Histopathological examination revealed a deposit of homogenous eosinophilic material throughout the dermis consistent with amyloidosis. Immunohistochemical study showed these deposits to be AL immunoglobin light chains., Discussion: The association of cutaneous nodular amyloidosis and Sjögren's syndrome appears to be a distinct disease entity reflecting the polymorphic clinical spectrum of lymphoproliferative diseases related to Sjögren's syndrome., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

IgG4-related disease is an emerging pathological condition characterized by one or several fibrosing and inflammatory organ involvements. Histological findings are typical and associate storiform fibrosis with polyclonal lymphocytic and plasma-cell infiltrate, with predominant IgG4-expressing plasma cells. Sclerosing lymphoplasmocytic pancreatitis, or type 1 auto-immune pancreatitis, and other organ involvements have been reported: sclerosing cholangitis, sialadenitis, dacryoadenitis, retroperitoneal fibrosis, aortitis, interstitial nephritis, polyadenopathy and inflammatory pseudo-tumors. Serum IgG4 level is elevated in most of patients, but the histological documentation remain necessary for the diagnosis. Dramatic response to steroids is usual but relapses are frequent.

Introduction: Central neurological manifestations of primary Sjogren's syndrome are very polymorphic, including pachymeningitis, one of the rarest manifestations, which is exceptionally inaugural., Clinical Case: A 50-year-old patient was admitted for an intracranial hypertension syndrome with paralysis of the III and VII cranial nerves. Brain MRI revealed pachymeningitis of the right cerebral hemisphere. History taking revealed the existence of xerostomy and xerophthalmos. The accessory salivary gland biopsy demonstrated Chisholm stage III sialadenitis. Search for anti-SSA antibodies and anti-SSB antibodies was positive. The diagnosis of primary Sjogren's syndrome was retained. The patient improved with corticosteroid therapy and cyclophosphamide., Conclusion: This case illustrates the unusual observation of pachymeningitis in primary Sjogren's syndrome in the rare setting as an inaugural manifestation., (Copyright © 2011. Published by Elsevier Masson SAS.)

Various systemic diseases can affect salivary glands. After a short anatomical and semiological recall, we describe the pathologies according to their possible salivary demonstrations, and we propose in a table a useful memorandum in daily practice.

ACCEPTED ASSOCIATIONS: Extrahepatic manifestations of the hepatitis C virus (HCV) have been reported in a large body of literature. It is now generally accepted that the HCV is strongly associated with so-called essential cryoglobulinemia, membrane proliferative glomerulonephritis with or without cryoglobulinemia, sporadic porphyria cutanea tarda, and with lymphocyte sialadenitis with or without a dry mouth syndrome. PROBABLE ASSOCIATIONS: Several epidemiological arguments suggest that HCV could play a role in certain types of non-Hodgkin lymphomas. HCV is also sometimes implicated in periarteritis nodosa and with peripheral neuropathies with lymphocyte vasculitis. UNPROVEN ASSOCIATIONS: The role of HCV in autoimmune dysthyroidism, lichen planus, diabetics mellitus, and antiphospholipid syndromes is a subject of debate. There have also many sporadic reports of manifestations of syndromes suggesting, though not proving, an association with the hepatitis C virus.

Investigation in 16 patients attending the stomatology outpatients clinic over the last 18 years has enabled definition of a particular radioclinical entity that has been named "idiopathic bilateral salivary megacanal". This generally parotid affection occurs in patients between 50 and 60 years, the onset usually being by an episode of sialodochitis. In addition to these inflammatory attacks there is the persistence of a bilateral "salivary ejaculation" from the ostia of the principal glands, sometimes after manual expression of a mucoid plug. Sialography shows dilatation of canals, the appearance being very suggestive for Stensen's duct, which becomes sinuous, enrolled and segmented. Dilatation is bilateral and affects the total length of the duct. The problem arises as to whether etiopathogenicity is from parietal canalar dysplasia or a constitutional anomaly of the nerve plexus comparable with that described in congenital megacolon. The latter hypothesis is plausible but requires confirmation from histopathology. The always benign course of the disease justifies conservative therapy only: oral antibiotics and canal massage several times a day, with a neostomy only when absolutely necessary.

On the strength of three new cases, it is suggested that the term "calcifying parotiditis" must be replaced by "salivary calcinosis". This suggestion is based on clinical and pathological grounds. An analysis of 11 cases of salivary calcinosis encountered in the last eight years points to clinical analogies between this condition and Gougerot-Sjögren's disease as well as to the fairly frequent involvement of the immune system in the calcinoses.